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pediatric epilepsy

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https://www.readbyqxmd.com/read/28101805/ketogenic-diet-in-pyruvate-dehydrogenase-complex-deficiency-short-and-long-term-outcomes
#1
Kalliopi Sofou, Maria Dahlin, Tove Hallböök, Marie Lindefeldt, Gerd Viggedal, Niklas Darin
OBJECTIVES: Our aime was to study the short- and long-term effects of ketogenic diet on the disease course and disease-related outcomes in patients with pyruvate dehydrogenase complex deficiency, the metabolic factors implicated in treatment outcomes, and potential safety and compliance issues. METHODS: Pediatric patients diagnosed with pyruvate dehydrogenase complex deficiency in Sweden and treated with ketogenic diet were evaluated. Study assessments at specific time points included developmental and neurocognitive testing, patient log books, and investigator and parental questionnaires...
January 18, 2017: Journal of Inherited Metabolic Disease
https://www.readbyqxmd.com/read/28101769/childhood-absence-epilepsy-and-benign-epilepsy-with-centro-temporal-spikes-a-narrative-review-analysis
#2
REVIEW
Alberto Verrotti, Renato D'Alonzo, Victoria Elisa Rinaldi, Sara Casciato, Alfredo D'Aniello, Giancarlo Di Gennaro
BACKGROUND: Recent studies have shown a possible coexistence of absence seizures with other forms of epilepsy. The purpose of this study was to ascertain the possible contemporary or subsequent presence of childhood absence epilepsy (CAE) and benign epilepsy with centro-temporal spikes (BECTS) in pediatric epileptic patients. METHODS: A PubMed systematic search indexed for MEDLINE, PubMed and EMBASE was undertaken to identify studies in children including articles written between 1996 and 2015...
January 19, 2017: World Journal of Pediatrics: WJP
https://www.readbyqxmd.com/read/28101478/focal-epileptogenic-lesions-in-adult-patients-with-epilepsy-and-generalized-epileptiform-discharges
#3
Dong Wook Kim, Seo-Young Lee, Sang Kun Lee
BACKGROUND AND PURPOSE: There are reports of successful resective epilepsy surgery for pediatric patients with epilepsy and generalized epileptiform discharges when they had focal epileptogenic lesions identified by MRI. However, there is limited information regarding adult patients with epilepsy who have both generalized epileptiform discharges and focal epileptogenic lesions. METHODS: To investigate the incidence and characteristics of adult patients who have both generalized epileptiform discharges and potentially epileptogenic lesions, we retrospectively analyzed data of clinical features and results of EEG and MRI of all patients with adult-onset epilepsy in a tertiary referral hospital...
December 2016: Journal of Epilepsy Research
https://www.readbyqxmd.com/read/28098941/posterior-cortex-epilepsy-surgery-in-childhood-and-adolescence-predictors-of-long-term-seizure-outcome
#4
Georgia Ramantani, Angeliki Stathi, Armin Brandt, Karl Strobl, Susanne Schubert-Bast, Gert Wiegand, Rudolf Korinthenberg, Vera van Velthoven, Josef Zentner, Andreas Schulze-Bonhage, Thomas Bast
OBJECTIVE: We aimed to investigate the long-term seizure outcome of children and adolescents who were undergoing epilepsy surgery in the parietooccipital cortex and determine their predictive factors. METHODS: We retrospectively analyzed the data of 50 consecutive patients aged 11.1 (mean) ± 5.1 (standard deviation) years at surgery. All patients but one had a magnetic resonance imaging (MRI)-visible lesion. Resections were parietal in 40%, occipital in 32%, and parietooccipital in 28% cases; 24% patients additionally underwent a resection of the posterior border of the temporal lobe...
January 18, 2017: Epilepsia
https://www.readbyqxmd.com/read/28089368/the-transition-from-pediatric-to-adult-care-for-youth-with-epilepsy-basic-biological-sociological-and-psychological-issues
#5
REVIEW
Peter Camfield, Carol Camfield, Kanetee Busiah, David Cohen, Alison Pack, Rima Nabbout
Transition from pediatric to adult health care for adolescents with epilepsy is challenging for the patient, family, and health care workers. This paper is the first of three that summarize the main findings from the 2nd Symposium on Transition in Epilepsies, held in Paris from June 14-25, 2016. In this paper we describe five basic themes that have an important effect on transition. First, there are important brain changes in adolescence that leave an imbalance between risk taking and pleasure seeking behaviors and frontal executive function compared with adults...
January 12, 2017: Epilepsy & Behavior: E&B
https://www.readbyqxmd.com/read/28088049/sudden-unexpected-death-in-epilepsy-sudep-disclosure-in-pediatric-epilepsy-an-italian-survey-on-to-tell-or-not-to-tell
#6
Galli Federica, Vignoli Aglaia, Canevini Maria Paola, Cerioli Gabriele, Vegni Elena
BACKGROUND AND OBJECTIVES: Although there has recently been significant debate regarding the importance of disclosing the risk of SUDEP, professional societies and clinical practice guidelines currently recommend that the risk of SUDEP be disclosed as part of a comprehensive epilepsy education program. Therefore, the primary aim of the present study was to examine whether healthcare providers treating pediatric patients with epilepsy in Italy would disclose the risk of SUDEP to the parents of children with epilepsy...
January 11, 2017: Epilepsy & Behavior: E&B
https://www.readbyqxmd.com/read/28087825/incidence-and-predictors-of-epilepsy-after-pediatric-arterial-ischemic-stroke
#7
Lori L Billinghurst, Lauren A Beslow, Nicholas S Abend, Michael Uohara, Laura Jastrzab, Daniel J Licht, Rebecca N Ichord
OBJECTIVE: To determine the cumulative incidence and clinical predictors of remote symptomatic seizures and epilepsy after pediatric arterial ischemic stroke (AIS). METHODS: We performed a retrospective analysis of 218 participants with neonatal AIS (NAIS), presumed perinatal AIS (PPAIS), and childhood AIS (CAIS) from a single-center prospective consecutive cohort enrolled from 2006 to 2014. Medical records were reviewed for timing, semiology, and treatment of acute symptomatic seizures, remote symptomatic seizures (RSS), and epilepsy...
January 13, 2017: Neurology
https://www.readbyqxmd.com/read/28078877/congenital-muscular-dystrophy-and-epilepsy-a-prospective-case-series-of-pediatric-patients
#8
G Vitaliti, P Pavone, C Romano, M Barbagallo, M Vecchio, C Ledda, R Lubrano, R Falsaperla
Congenital Muscular Dystrophies (CMDs) can be considered as a heterogeneous group of diseases characterized by marked weakness, generalized hypotonia and joint contractures. They are divided into pure and classical forms, without ocular and cerebral involvement, and complex forms, which are associated with cerebral abnormalities. Seizures have rarely been described in the pure forms while they seem to occur more frequently in complex forms. The aim of our study was to evaluate the incidence of seizure in CMD...
October 2016: Journal of Biological Regulators and Homeostatic Agents
https://www.readbyqxmd.com/read/28062149/prediction-of-neonatal-seizures-in-hypoxic-ischemic-encephalopathy-using-electroencephalograph-power-analyses
#9
Siddharth V Jain, Amit Mathur, Preethi Srinivasakumar, Michael Wallendorf, Joseph P Culver, John M Zempel
BACKGROUND: The severity of the initial encephalopathy in neonatal hypoxic-ischemic encephalopathy correlates with seizure burden. Early electroencephalograph (EEG) background activity reflects the severity of encephalopathy. Thus, we hypothesized that early EEG background would be predictive of subsequent seizures in neonatal hypoxic-ischemic encephalopathy. METHODS: This study included infants undergoing therapeutic hypothermia at St. Louis Children's Hospital between January 2009 and April 2013...
November 11, 2016: Pediatric Neurology
https://www.readbyqxmd.com/read/28060325/interictal-high-frequency-oscillations-detected-with-simultaneous-magnetoencephalography-and-electroencephalography-as-biomarker-of-pediatric-epilepsy
#10
Christos Papadelis, Eleonora Tamilia, Steven Stufflebeam, Patricia E Grant, Joseph R Madsen, Phillip L Pearl, Naoaki Tanaka
Crucial to the success of epilepsy surgery is the availability of a robust biomarker that identifies the Epileptogenic Zone (EZ). High Frequency Oscillations (HFOs) have emerged as potential presurgical biomarkers for the identification of the EZ in addition to Interictal Epileptiform Discharges (IEDs) and ictal activity. Although they are promising to localize the EZ, they are not yet suited for the diagnosis or monitoring of epilepsy in clinical practice. Primary barriers remain: the lack of a formal and global definition for HFOs; the consequent heterogeneity of methodological approaches used for their study; and the practical difficulties to detect and localize them noninvasively from scalp recordings...
December 6, 2016: Journal of Visualized Experiments: JoVE
https://www.readbyqxmd.com/read/28056630/a-model-program-for-translational-medicine-in-epilepsy-genetics
#11
Lacey A Smith, Jeremy F P Ullmann, Heather E Olson, Christelle M El Achkar, Gessica Truglio, McKenna Kelly, Beth Rosen-Sheidley, Annapurna Poduri
Recent technological advances in gene sequencing have led to a rapid increase in gene discovery in epilepsy. However, the ability to assess pathogenicity of variants, provide functional analysis, and develop targeted therapies has not kept pace with rapid advances in sequencing technology. Thus, although clinical genetic testing may lead to a specific molecular diagnosis for some patients, test results often lead to more questions than answers. As the field begins to focus on therapeutic applications of genetic diagnoses using precision medicine, developing processes that offer more than equivocal test results is essential...
January 1, 2017: Journal of Child Neurology
https://www.readbyqxmd.com/read/28049367/sensitivity-of-the-wisconsin-card-sorting-test-64-card-version-versus-the-tower-of-london-drexel-version-for-detecting-executive-dysfunction-in-children-with-epilepsy
#12
William S MacAllister, Moshe Maiman, Megan Marsh, Lindsay Whitman, Marsha Vasserman, Robyn J Cohen, Christine M Salinas
Executive function deficits are common in children and adolescents with epilepsy. Though the Wisconsin Card Sorting Task (WCST) is often considered the "gold standard" for executive function assessment, its sensitivity-particularly in the case of the 64-card version (WCST-64)-is insufficiently established in pediatric samples, including children and adolescents with epilepsy. The present investigation assesses the sensitivity of the WCST-64 in children and adolescents with epilepsy in comparison to another measure: the Tower of London - Drexel Version (TOL-DX)...
January 3, 2017: Child Neuropsychology: a Journal on Normal and Abnormal Development in Childhood and Adolescence
https://www.readbyqxmd.com/read/28041798/early-seizures-predict-the-development-of-epilepsy-in-children-and-adolescents-with-stroke
#13
Ina Breitweg, Celina von Stülpnagel, Tom Pieper, Karen Lidzba, Hans Holthausen, Martin Staudt, Gerhard Kluger
PURPOSE: To identify risk factors for the development of epilepsy after pediatric stroke. METHODS: Retrospective analysis of hospital charts of 93 children and adolescents with post-neonatal non-traumatic stroke and a minimum follow-up of two years. Seizures during the first 48 h after onset of stroke symptoms were defined as "early seizures"; when two or more seizures occurred after this period, the patient was classified as "epileptic". RESULTS: Early seizures, young age at stroke and MRI evidence of cortical involvement were observed more frequently in the children who developed epilepsy...
December 18, 2016: European Journal of Paediatric Neurology: EJPN
https://www.readbyqxmd.com/read/28040557/measurement-of-pyridoxal-5-phosphate-pyridoxal-and-4-pyridoxic-acid-in-the-cerebrospinal-fluid-of-children
#14
Tomoyuki Akiyama, Mari Akiyama, Yumiko Hayashi, Takashi Shibata, Yoshiyuki Hanaoka, Soichiro Toda, Katsumi Imai, Shin-Ichiro Hamano, Tohru Okanishi, Harumi Yoshinaga, Katsuhiro Kobayashi
BACKGROUND: We quantified pyridoxal 5'-phosphate (PLP), pyridoxal (PL), and 4-pyridoxic acid (PA) in the cerebrospinal fluid (CSF) of children and to investigate the effect of age, sex, epilepsy, and anti-epileptic drug (AED) therapy on these vitamers. METHODS: CSF samples prospectively collected from 116 pediatric patients were analyzed. PLP, PL, and PA were measured using high-performance liquid chromatography with fluorescence detection, using pre-column derivatization by semicarbazide...
December 28, 2016: Clinica Chimica Acta; International Journal of Clinical Chemistry
https://www.readbyqxmd.com/read/28038823/a-kcnq2-e515d-mutation-associated-with-benign-familial-neonatal-seizures-and-continuous-spike-and-waves-during-slow-wave-sleep-syndrome-in-taiwan
#15
Inn-Chi Lee, Jiann-Jou Yang, Shuan-Yow Li
BACKGROUND/PURPOSE: Pediatric epilepsy caused by a KCNQ2 gene mutation usually manifests as benign familial neonatal seizures (BFNS) during the 1(st) week of life. However, the exact mechanism, phenotype, and genotype of the KCNQ2 mutation are unclear. METHODS: We studied the KCNQ2 genotype from 75 nonconsanguineous patients with childhood epilepsy without an identified cause (age range: from 2 days to 18 years) and from 55 healthy adult controls without epilepsy...
December 27, 2016: Journal of the Formosan Medical Association, Taiwan Yi Zhi
https://www.readbyqxmd.com/read/28018437/1p36-deletion-syndrome-confirmed-by-fluorescence-in-situ-hybridization-and-array-comparative-genomic-hybridization-analysis
#16
Dong Soo Kang, Eunsim Shin, Jeesuk Yu
Pediatric epilepsy can be caused by various conditions, including specific syndromes. 1p36 deletion syndrome is reported in 1 in 5,000-10,000 newborns, and its characteristic clinical features include developmental delay, mental retardation, hypotonia, congenital heart defects, seizure, and facial dysmorphism. However, detection of the terminal deletion in chromosome 1p by conventional G-banded karyotyping is difficult. Here we present a case of epilepsy with profound developmental delay and characteristic phenotypes...
November 2016: Korean Journal of Pediatrics
https://www.readbyqxmd.com/read/28011930/refractory-status-epilepticus-in-children-with-and-without-prior-epilepsy-or-status-epilepticus
#17
Iván Sánchez Fernández, Michele C Jackson, Nicholas S Abend, Ravindra Arya, James N Brenton, Jessica L Carpenter, Kevin E Chapman, William D Gaillard, Marina Gaínza-Lein, Tracy A Glauser, Joshua L Goldstein, Howard P Goodkin, Ashley Helseth, Kush Kapur, Tiffani L McDonough, Mohamad A Mikati, Katrina Peariso, James Riviello, Robert C Tasker, Alexis A Topjian, Mark S Wainwright, Angus Wilfong, Korwyn Williams, Tobias Loddenkemper
OBJECTIVE: To compare refractory convulsive status epilepticus (rSE) management and outcome in children with and without a prior diagnosis of epilepsy and with and without a history of status epilepticus (SE). METHODS: This was a prospective observational descriptive study performed from June 2011 to May 2016 on pediatric patients (1 month-21 years of age) with rSE. RESULTS: We enrolled 189 participants (53% male) with a median (25th-75th percentile) age of 4...
December 23, 2016: Neurology
https://www.readbyqxmd.com/read/28010956/central-tegmentum-tract-hyperintensities-in-pediatric-neurological-patients-incidence-or-coincidence
#18
Uğur Işık, Alp Dinçer
AIM: The central tegmental tract hyperintensities (CTTH) have been found in many different pediatric neurological conditions. There is only scarce data about the value of this radiological phenomenon. In this study we aimed to show the neurological conditions associated with this radiological finding. MATERIALS AND METHODS: We performed a retrospective analysis of all pediatric brain MRI's between 2013 and 2015. After finding those patients with CTTH, we evaluated them in the pediatric neurology clinic...
December 20, 2016: Brain & Development
https://www.readbyqxmd.com/read/28004629/manifestations-of-tuberous-sclerosis-complex-the-experience-of-a-provincial-clinic
#19
Colin Wilbur, Chinnuwat Sanguansermsri, Hedi Chable, Mihaela Anghelina, Sharon Peinhof, Kelly Anderson, Paul Steinbok, Ash Singhal, Anita Datta, Mary B Connolly
BACKGROUND: Tuberous sclerosis complex (TSC) is a neurocutaneous disorder with a wide spectrum of manifestations. Recent consensus recommendations stress the importance of multidisciplinary management of children with TSC. The objective of this study was to examine the manifestations of TSC at a large referral centre to determine the care needs of this population. METHODS: A retrospective, systematic chart review was performed of children with TSC managed at British Columbia Children's Hospital...
January 2017: Canadian Journal of Neurological Sciences. le Journal Canadien des Sciences Neurologiques
https://www.readbyqxmd.com/read/28003958/metrics-of-brain-network-architecture-capture-the-impact-of-disease-in-children-with-epilepsy
#20
Michael J Paldino, Wei Zhang, Zili D Chu, Farahnaz Golriz
BACKGROUND AND OBJECTIVE: Epilepsy is associated with alterations in the structural framework of the cerebral network. The aim of this study was to measure the potential of global metrics of network architecture derived from resting state functional MRI to capture the impact of epilepsy on the developing brain. METHODS: Pediatric patients were retrospectively identified with: 1. Focal epilepsy; 2. Brain MRI at 3 Tesla, including resting state functional MRI; 3. Full scale IQ measured by a pediatric neuropsychologist...
2017: NeuroImage: Clinical
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